Keratoconus Causes, Symptoms And 10 Treatment Options

Keratoconus is a progressive eye disease in which the normally round cornea thins and begins to bulge into a cone-like shape. This cone shape deflects light as it enters the eye on its way to the light-sensitive retina, causing distorted vision.

Keratoconus can occur in one or both eyes and often begins during a person's teens or early 20s.

Keratoconus Symptoms And Signs

As the cornea becomes more irregular in shape, it causes progressive nearsightedness and irregular astigmatism to develop, creating additional problems with distorted and blurred vision. Glare and light sensitivity also may occur.

What Causes Keratoconus?

New research suggests the weakening of the corneal tissue that leads to keratoconus may be due to an imbalance of enzymes within the cornea. This imbalance makes the cornea more susceptible to oxidative damage from compounds called free radicals, causing it to weaken and bulge forward.

Risk factors for oxidative damage and weakening of the cornea include a genetic predisposition, explaining why keratoconus often affects more than one member of the same family.

Keratoconus Treatment

In the mildest form of keratoconus, eyeglasses or soft contact lenses may help. But as the disease progresses and the cornea thins and becomes increasingly more irregular in shape, glasses and regular soft contact lens designs no longer provide adequate vision correction.

With epithelium-off crosslinking, the outer layer of the cornea (called the epithelium) is removed to allow entry of riboflavin, a type of B vitamin, into the cornea, which then is activated with UV light.

With the epithelium-on method (also called transepithelial crosslinking), the corneal epithelium is left intact during the treatment. The epithelium-on method requires more time for the riboflavin to penetrate into the cornea, but potential advantages include less risk of infection, less discomfort and faster visual recovery, according to supporters of this technique.

And Dr. Brian Boxer Wachler, who began performing and developing transepithelial crosslinking in 2003, has reported excellent results with keratoconus patients such as bobsledder Steve Holcomb, whose improved vision helped his team win a gold medal in the 2010 Winter Olympics. (Watch video.)

January 2016  Thanks to vast new insurance data troves that allow researchers to evaluate characteristics associated with various diseases, doctors may be able to detect keratoconus sooner. This would allow more patients to benefit from new treatments aimed at slowing disease progression and protecting vision.

Men, Latinos and certain other groups are more likely to develop keratoconus than others.

Sidebar continued >>

Researchers at the University of Michigan Health System's Kellogg Eye Center and the U-M Institute for Healthcare Policy and Innovation reviewed the health insurance claims of more than 32,000 people, half of whom had confirmed keratoconus.

This was the largest keratoconus study ever conducted, and it revealed several risk factors that are now known to be associated with keratoconus.

In the study, men, African-Americans and Latinos, as well as people with asthma, sleep apnea or Down syndrome, had much higher odds of developing keratoconus. In fact, people with Down syndrome were six times more likely to have keratoconus; and keratoconus prevalence in people of African American and Latino origin was 50 percent higher than it was in whites.

Anyone who falls into one of these groups or who has sleep apnea or asthma is at increased risk, the study found.

Although there are new treatments for keratoconus, people often don't receive a diagnosis early enough to take full advantage of them. But thanks to this new research, doctors are likely to take a closer look at corneal shape in these at-risk groups, so that treatment can be initiated sooner and vision loss due to keratoconus can be minimized.  A.H.

In April 2016, the FDA granted approval of Avedro's KXL System corneal collagen cross-linking treatment of progressive keratoconus. The approval includes use of the pharmaceutical and medical device company's Photrexa and Photrexa Viscous riboflavin solutions with the KXL System during the procedure.

Avedro's Photrexa/KXL System is the first corneal collagen cross-linking system to gain the required regulatory approval to be performed by eye doctors in the United States for this sight-threatening condition. More than 200,000 patient eyes have been treated with the system outside the United States prior to FDA approval, according to the company.

Corneal crosslinking may reduce significantly the need for corneal transplants among keratoconus patients. It also is being investigated as a way to treat or prevent complications following LASIK or other vision correction surgery.

Using a combination of corneal crosslinking and Intacs implants also has demonstrated promising results for treating keratoconus. Also, progressive mild to moderate keratoconus has been safely and successfully treated with a combination of corneal crosslinking and implantation of a toric phakic IOL.

Custom soft contact lenses. Recently, contact lens manufacturers have introduced custom soft contact lenses specially designed to correct mild-to-moderate keratoconus. These lenses are made-to-order based on detailed measurements of the person's keratoconic eye(s) and may be more comfortable than gas permeable lenses (GPs) or hybrid contact lenses for some wearers.

In the United States, custom soft contacts that are available for the correction of keratoconus include:

KeraSoft lenses (Bausch + Lomb). These high-water silicone hydrogel lenses can correct up to 20 diopters (D) of nearsightedness or farsightedness and up to -12 D of astigmatism.

NovaKone lenses (Bausch + Lomb). These medium-water hydrogel lenses can correct up to 30 D of nearsightedness or farsightedness and up to -10 D of astigmatism.

Both lenses have a very wide range of fitting parameters for a customized fit and are larger in diameter than regular soft lenses for greater stability on a keratoconic eye.

In a recent study of the visual performance of toric soft contacts and rigid gas permeable lenses for the correction of mild keratoconus, though GP lenses provided better visual acuity in low-contrast situations, soft toric lenses performed equally well in high-contrast acuity testing.

Custom toric soft contacts for keratoconus are significantly more expensive than regular soft contacts, but vision insurance may cover some of the costs associated with contact lenses for keratoconus.

Gas permeable contact lenses. If eyeglasses or soft contact lenses cannot control keratoconus, then gas permeable contact lenses usually are the preferred treatment. GP lenses vault over the cornea, replacing its irregular shape with a smooth, uniform refracting surface to improve vision.

Fitting contact lenses on an eye with keratoconus often is challenging and time-consuming. You can expect frequent return visits to your eye care provider so he or she can fine-tune the fit and your prescription, especially if your keratoconus continues to progress.

Treatments for keratoconus include specially designed contacts and surgery.

"Piggybacking" contact lenses. Because fitting a gas permeable contact lens over a cone-shaped cornea can sometimes be uncomfortable for a person with keratoconus, some eye care practitioners advocate "piggybacking" two different types of contact lenses on the same eye.

For keratoconus, this method involves placing a soft contact lens, such as one made of silicone hydrogel, over the eye and then fitting a GP lens over the soft lens. This approach increases wearer comfort because the soft lens acts like a cushioning pad under the rigid GP lens.

Your eye care practitioner will monitor closely the fitting of "piggyback" contact lenses to make sure enough oxygen reaches the surface of your eye, which can be a problem when two lenses are worn on the same eye. However, most modern contacts  both GP and soft  typically have adequate oxygen permeability for a safe "piggyback" fit.

Hybrid contact lenses. The UltraHealth and ClearKone hybrid contact lenses manufactured by SynergEyes combine a highly oxygen-permeable rigid center with a soft peripheral "skirt." These lenses were designed specifically for keratoconus, and the central GP zone of the lens vaults over the cone-shaped cornea for increased comfort.

Hybrid contact lenses provide the crisp optics of a gas permeable contact lens and wearing comfort that rivals that of soft lenses, according to SynergEyes. UltraHealth and ClearKone are available in a wide variety of parameters to provide a fit that conforms well to the irregular shape of a keratoconic eye.

Keratoconus News

Corneal Collagen Crosslinking Stabilizes Eyes Of Children With Keratoconus

August 2015  Corneal collagen crosslinking (CXL), a relatively non-invasive procedure that uses topical application of riboflavin (vitamin B2) and UV light to strengthen chemical bonds in the cornea, effectively stabilized the eyes of children with keratoconus, according to a recent study.

Corneal crosslinking may help kids with keratoconus.

Sidebar continued >>

Researchers at Temple Street Children's University Hospital in Dublin, Ireland, evaluated the medical records of children (ages 13 to 18 years) with keratoconus who had undergone CXL. A total of 25 eyes of 14 patients were treated and evaluated.

Measurements of visual acuity, mean refractive error, amount of astigmatism and corneal curvature were taken before treatment and three, six and 12 months afterward.

Prior to treatment, mean uncorrected visual acuity (UCVA) was approximately 20/70, and mean best-corrected visual acuity (BCVA) with eyeglasses was 20/40. One year after CXL, mean UCVA was approximately 20/60 and mean BCVA improved to 20/30.

Measurements of mean refractive error, amount of astigmatism, and corneal curvature mapping (topography) before and one year after the procedure remained stable, and there were no significant complications.

According to the study authors, CXL effectively stabilized the refractive error and corneal shape of children with keratoconus, while improving their best-corrected visual acuity.

A full report of the study was published in the June issue of Journal of the American Association for Pediatric Ophthalmology and Strabismus (JAAPOS).  G.H.

Scleral and semi-scleral lenses. These are large-diameter gas permeable contacts  large enough that the periphery and edge of the lens rest on the "white" of the eye (sclera). Scleral lenses cover a larger portion of the sclera, whereas semi-scleral lenses cover a smaller area.

Because the center of scleral and semi-scleral lenses vaults over the irregularly shaped cornea, these lenses don't apply pressure to the eye's cone-shaped surface for a more comfortable fit. These larger lenses also are more stable than conventional gas permeable contact lenses, which move with each blink because they cover only a portion of the cornea.

Prosthetic lenses. Because keratoconic eyes are so challenging to fit, patients with severe disease often require an advanced scleral lens design that doubles as a prosthetic shell.

One example of a prosthetic lens is the BostonSight PROSE, a prosthetic replacement of the ocular surface ecosystem that's been in use since the 1990's. This treatment restores visual function, supports healing, reduces symptoms and improves quality of life for patients suffering with complex corneal disease such as keratoconus.

To qualify for PROSE, you must have a severe, unusual or otherwise untreatable condition. In cases of financial need, the non-profit Boston Foundation for Sight, which developed PROSE, may help subsidize the cost of the fitting and lens, which typically runs several thousand dollars. Also, PROSE is currently only available at a few top-ranked academic medical centers.

A second type of lens is the EyePrintPRO (EyePrint Prosthetics). This prosthetic scleral cover shell is designed utilizing Elevation Specific Technology to exactly match the unique irregularities of each individual eye. The process begins with an EyePrint Impression, which captures the precise curvatures of the entire ocular surface. Doctors then send the resulting impression to an EyePrint lab for 3-D scanning. The scanned data is then fed to a numerically controlled machining system to produce a lens that matches the impression exactly and therefore fits the individual cornea and sclera perfectly. Because of the precise nature of the back surface fit, high quality and individualized optics can be placed on the front surface of the device.

"The EyePrint is like a fingerprintit's completely individual," says EyePrint developer Dr. Christine Sindt, Clinical Associate Professor of Ophthalmology and Visual Sciences at University of Iowa.

The EyePrintPRO can only be fit by doctors who undergo specific training and certification. However, the device is now available at nearly 50 practices throughout the US and Canada.

Intacs. These surgically applied corneal inserts manufactured by Addition Technology received FDA approval for treating keratoconus in August 2004. The tiny plastic inserts are placed just under the eye's surface in the periphery of the cornea and help reshape the cornea for clearer vision.

Intacs may be needed when keratoconus patients no longer can obtain functional vision with contact lenses or eyeglasses.

Several studies show that Intacs can improve the best spectacle-corrected visual acuity (BSCVA) of a keratoconic eye by an average of two lines on a standard eye chart. The implants also have the advantage of being removable and exchangeable. The surgical procedure takes only about 10 minutes.

Topography-guided conductive keratoplasty. While more study is needed, early results of a small study involving topography-guided conductive keratoplasty (CK) show this procedure might help smooth irregularities in the corneal surface.

This treatment uses energy from radio waves, applied with a small probe at several points in the periphery of the cornea to reshape the eye's front surface. A topographic "map" created by computer imaging of the eye's surface helps create individualized treatment plans.

In October 2010, the American Journal of Ophthalmology reported that 15 of 21 keratoconic eyes treated with topography-guided CK achieved more normal corneal symmetry.

Corneal transplant. Some people with keratoconus can't tolerate a rigid contact lens, or they reach the point where contact lenses or other therapies no longer provide acceptable vision.

The last remedy to be considered may be a cornea transplant, also called a penetrating keratoplasty (PK or PKP). Even after a transplant, you most likely will need glasses or contact lenses for clear vision.

Visual performance and optical quality with soft lenses in keratoconus patients. Ophthalmic and Physiological Optics. Published online ahead of print, January 2012.

About the Reviewer: William Trattler, MD, is a board-certified ophthalmologist specializing in refractive, corneal and cataract eye surgery at the Center for Excellence in Eye Care  a private practice in Miami that treats a wide range of vision disorders and provides laser vision correction. More about Dr. Trattler